Challenges of anaesthetising a child with Bow Hunter's syndrome and dilated cardiomyopathy for occipitocervical fusion

BMJ Case Rep. 2023 May 2;16(5):e253834. doi: 10.1136/bcr-2022-253834.


Paediatric Bow Hunter's syndrome (BHS), or rotational vertebral artery syndrome, is a rare cause of posterior circulation insufficiency in children. It results from mechanical obstruction of the vertebral artery by the transverse process of cervical vertebrae resulting in vertebrobasilar insufficiency during the neck rotation to the sides. Paediatric dilated cardiomyopathy (DCM) is a rare myocardial disease that presents with ventricular dilatation and cardiac dysfunction. This case report describes the successful anaesthetic management of an boy with BHS due to atlantoaxial dislocation and DCM. The child was anaesthetised by keeping the following anaesthetic goals in mind such as maintenance of the heart rate, rhythm, preload, afterload and contractility close to the baseline for both DCM and BHS. Haemodynamic management with optimal fluids, inotrope and a vasopressor and titrating its volume and doses using multimodal haemodynamic monitoring while keeping both cardio and neuroprotective strategies, and the multimodal analgesia techniques helped the child for faster recovery.

Keywords: Heart failure; Neuroanaesthesia; Neurosurgery; Paediatrics.

Publication types

  • Case Reports

MeSH terms

  • Cardiomyopathy, Dilated* / complications
  • Cardiomyopathy, Dilated* / surgery
  • Child
  • Humans
  • Male
  • Mucopolysaccharidosis II* / complications
  • Spinal Fusion* / adverse effects
  • Vertebral Artery
  • Vertebrobasilar Insufficiency* / etiology